A 36-year-old man, who was referred for severe chest pains, was found to have acute type II aortic dissection associated with a dilated ascending aorta, aortic coarctation, and congenitally bicuspid valve. A single-stage surgical repair consisting of valve-sparing aortic root implantation and graft replacement of the ascending aorta, arch, and the coarctation segment was successful.